Autoimmune Diseases Skin

Skin Disease Directory

Overview

Scleroderma is a rare autoimmune disease that often affects the skin. There are over 3,000 skin diseases, and some of them share similar symptoms with scleroderma. Here we focus on the conditions that are more commonly confused with scleroderma or that occasionally overlap with it. (Also see: Autoimmune Disease Directory and What is Scleroderma?)

Histopathology of the skin in rheumatic diseases. In this review, we discuss the clinical features, diagnostic evaluation and the histopathological features of skin manifestation of the most relevant rheumatological autoimmune diseases. PubMed, Reumatismo, 2018 Oct 3;70(3):187-198.

Acrokeratoelastoidosis

Acrokeratoelastoidosis (AKE) is a rare genodermatosis characterized by small, firm papules or plaques on the sides of the hands and feet. These nodules may result from an abnormality in the secretion or excretion of elastic material by fibroblasts in the dermis. Medscape.

Atrophoderma of Pierini and Pasini

Atrophoderma of Pierini and Pasini APP) is similar to Morphea Scleroderma. Some doctors think it represents a late-stage Morphea. ISN.

Bullous Pemphigoid

Bullous Pemphigoid. American Osteopathic College of Dermatology.

Bullous Pemphigoid. Bullous pemphigoid is a skin disorder characterized by large blisters. Medline Plus.

Dermatitis Artefacta or Factitial Dermatitis

Dermatitis Artefacta means that somebody has injured their own skin, by any method. ISN.

Eosinophilic Fasciitis (Shulman's Syndrome)

Eosinophilic Fasciitis The debate is ongoing as to whether or not eosinophilic fasciitis is a form of scleroderma. ISN.

Eosinophilia-Myalgia Syndrome

Eosinophilia-Myalgia Syndrome (EMS) is a multi-systemic disease caused by ingestion of impure L-tryptophan, an amino acid dietary supplement.

Graft-versus-Host Disease (GVHD)

Graft-versus-Host Disease (GVHD) is an autoimmune response to foreign cells, such as from skin grafting, bone marrow transplants, stem cell transplants, blood transfusions, or organ transplants.

Grzybowski Syndrome

Grzybowski generalized eruptive keratoacanthoma. Grzybowski syndrome, or generalized eruptive keratoacanthoma, refers to a very rare disease in which hundreds of keratoacanthoma-like papules appear. Grzybowski syndrome, or generalized eruptive keratoacanthoma, refers to a very rare disease in which hundreds of keratoacanthoma-like papules appear. DermNet NZ.

Keloidal Scleroderma

Keloids are smooth, shiny, flesh-colored, raised growths of fibrous tissue that form over areas of injury or surgical wounds. Keloidal Scleroderma is a rare, disfiguring variant of scleroderma. ISN.

Lichen Sclerosus or Lichen Sclerosus et Atrophicus

Lichen Sclerosus (aka Lichen Sclerosis or Lichen Sclerosus et Atrophicus) is a skin condition that affects the vulva (or penis) and anus. ISN.

Lipodermatosclerosis

Lipodermatosclerosis: a clinicopathologic correlation. Lipodermatosclerosis (LDS) is a chronic fibrosing panniculitis associated with venous insufficiency. PubMed, Int J Dermatol.

Lipodystrophy, Lipoatrophy

Morphea Scleroderma

Myeloma (Bone Marrow Cancer)

Myeloma: From Deadly to Chronic. Multiple myeloma (MM) is the second most commonly diagnosed blood cancer, after non-Hodgkin lymphoma, yet few people know much about this deadly disease. Celgene. (Also see Scleroderma and Cancer)

Nephrogenic Fibrosing Dermopathy or Nephrogenic Systemic Fibrosis (NSF)

Nephrogenic Systemic Fibrosis (NSF). Nephrogenic systemic fibrosis resembles scleroderma and eosinophilic fasciitis clinically and scleromyxedema histopathologically. Patients with NSF may develop large areas of indurated skin with fibrotic nodules and plaques. Medscape.

Overlap Syndrome

Scleroderma in Overlap. When Systemic Sclerosis (in either the Limited or the Diffuse form) appears in conjunction with features of one or more other connective tissue diseases (such as Systemic Lupus Erythematosus, Polymyositis, Dermatomyositis) it is called "overlap." ISN.

Parry Rombergs Syndrome

Parry Rombergs Syndrome, aka Progressive Hemifacial Atrophy or HFA. This may be the same as Linear Scleroderma, affecting the facial area. It usually affects the left half of the face and the facial atrophy may be accompanied by other symptoms, including migraine headaches and trigeminal neuralgia. ISN.

Pemphigus Vulgaris

What is Pemphigus Vulgaris? Pemphigus vulgaris is an autoimmune skin disorder characterized by blistering of the skin and mucous membrane. Medline Plus.

Perioral Dermatitis or Periorificial Dermatitis

Perioral Dermatitis (Also called periorificial dermatitis) is a facial rash that tends to occur around the mouth and/or nose and occasionally, the eyes. ISN.

POEMS

POEMS stands for Polyneuropathy, Organomegaly, Endocrinopathy, M-proteins, and Skin changes. The skin changes may be similar to Scleroderma. It is also known as Crow-Fukase Syndrome, PEP Syndrome, Polyeuropathy-organomegaly-endocrinopathy-M protein-skin lesions, Shimpo syndrome, Takatsuki syndrome, and Polyneuropathy-organomegaly-endocrinopathy-M protein-skin lesions.

Porphyria Cutanea Tarda (PCT)

For UVA1 treatment, first contact your doctor to see if it they will recommend it for you. It is not used for lichen sclerosus that is on the genital area. If they recommend and prescribe it for you, try to find UVA1 facilities near you. Some dermatology offices (usually large offices or with major health centers) have UVA1 equipment (not UVB). It may also be possible to buy UVA1 phototherapy equipment for home use. It is manufactured by Daavlin and distributed worldwide. Home users must be monitored by a physician, who will prescribe the necessary dosage and monitor for possible side-effects, such as skin cancer.

Progeria, Hutchinson-Gilford Progeria

What is Progeria? Progeria syndromes give the appearance of premature aging. Many people know progeria from seeing children who look very old on TV talk shows and documentaries. Those children have the more rare form of progeria; there is another more common type that affects adolescents and adults. VeryWell.

What is Hutchinson-Gilford Progeria Syndrome? It is a form of progeria that begins to show around 6 to 12 months of age, when the child begins to look like an elderly person. The life expectancy is about 13 years. VeryWell.

Pseudoscleroderma

Pseudoscleroderma refers to skin diseases that are similar to either systemic or localized scleroderma. A broad spectrum of unrelated disorders is included in this category, such as scleredema, diffuse fasciitis with blood eosinophilia, progeria, Werner's disease, carcinoid syndrome, chronic graft-versus-host disease, porphyria cutanea tarda, phenylketonuria, scleromyxoedema, scleroderma-like lesions due to bleomycin therapy, occupational sclerodermas and melorheostosis with linear scleroderma.

Psoriasis

Psoriasis and Psoriatic Arthritis. Psoriasis is an autoimmune skin disease that causes scaly red patches of skin. Sometimes people with psoriasis develop joint pain, which is called psoriatic arthritis. ISN.

Radiation Port Scleroderma

Causes of Scleroderma: Radiation There is an increased rate of cancer among scleroderma patients. Furthermore, scleroderma patients have an increased sensitivity to radiation treatments. In addition, radiation can cause scleroderma including Radiation Port Scleroderma.

Schnitzler Syndrome

Schnitzler syndrome (SS), first reported in 1972,1 is characterized by chronic, nonpruritic urticaria in association with recurrent fever, bone pain, arthralgia or arthritis, and a monoclonal immunoglobulin M (IgM) gammopathy in a concentration that is usually less than 10 g/L. Since 1972, approximately 100 cases of Schnitzler syndrome have been reported. Medscape.

Scleredema adultorum Buschke

Scleredema is an illness similar to scleroderma. It is also known as Scleredema Adultorum, Scleredema Adultorum of Buschke, Scleredema Diabeticorum, and Scleredema Diabeticorum of Buschke.

Scleroderma

Scleroderma-Like

(Case Report) Phaeochromocytoma Mimicking Scleroderma. Phaeochromocytoma should be suspected in patients with features of scleroderma who are negative for auto-antibodies. International Journal of Endocrinology. (Also see Scleroderma-Like)

(Case Report) Taxane-induced scleroderma. Although the mechanisms have not been clarified, it should be noted that taxane is causally involved in the formation of scleroderma-like skin conditions. PubMed, Br J Dermatol. (Also see Causes of Scleroderma: Drugs and Medications)

(Case Report) Reticulate hyperpigmented scleroderma: a new pigmentary manifestation. We report another unrecognized pigmentary abnormality in a 48-year-old patient who presented with a reticulate hyper-pigmentation affecting the trunk, upper and lower limbs. To our knowledge, cutaneous hyperpigmented reticulate scleroderma has not been reported previously. PubMed, Clin Exp Dermatol.

(Case Report) Gemcitabine-associated scleroderma-like changes of the lower extremities. Gemcitabine is a nucleosid analog approved for use in the treatment of metastatic urothelial carcinoma of the bladder. This is the first case report of a scleroderma-like reaction associated with gemcitabine. This antineoplastic agent must be added to the very limited number of cytostatic agents capable of giving rise to scleroderma-like features. PubMed, J Am Acad Dermatol. (Also see Causes of Scleroderma: Medications)

Donna S: Odd Version of Scleroderma My dermatologist has spoken with numerous dermatologists across the country, and they have not heard of my particular case of scleroderma. They also have not heard of blisters being associated with scleroderma in any form…

Scleromyxedema aka Lichen Myxedematosus or Papular Mucinosis

Diseases Similar to Scleroderma: Scleromyxedema is a scleroderma-like condition. However, it causes thickening and hardening that makes the skin look too ample, rather than too tight, as in scleroderma. It is also referred to as Scleromyxedema of Groton, lichen myxedematosus, and papular mucinosis. Overview, symptoms, diagnosis, treatments, support, and personal stories. ISN.

Stiff Skin Syndrome (SSS)

Stiff skin syndrome (SSS) is a rare condition characterized by hard, thick skin (usually on the entire body), which limits joint mobility and causes flexion contractures (when joints are stuck in a bent position). The condition may be apparent at birth, or may become apparent before age 6. NORD.

Urticaria

Urticaria. Urticaria may be confused with a variety of other dermatologic diseases that are similar in appearance and are pruritic including atopic dermatitis (eczema), maculopapular drug eruptions, contact dermatitis, insect bites, erythema multiforme, pityriasis rosea, and others. Medscape.

Vitiligo

Diseases Similar to Scleroderma: Vitiligo Includes overview, symptoms, causes, treatments, research and personal stories. ISN.

Warts

Warts. People with immune deficiencies are more susceptible to contracting the virus that causes warts. Common warts, genital warts, and plantar warts are all caused by different strains of the human papillomavirus. Warts.org.

Werner's Syndrome

Werner's Syndrome. Werner syndrome is characterized by the dramatic, rapid appearance of features associated with normal aging. Individuals with this disorder typically grow and develop normally until they reach puberty. Genetics Home Reference.

Footnotes

(1) Medscape requires (free) sign up and password. You do not need to be a medical professional to use Medscape. However this makes for slower access to the site for first-time users.

SCLERO.ORG is the world's leading nonprofit for trustworthy research, support, education and awareness for scleroderma and related illnesses. We are a 501(c)(3) U.S.-based public charitable foundation, established in 2002. Meet Our Team. Donations may also be mailed to: