[Increasing pigmentation in Schmidt syndrome (polyglandular autoimmune syndrome type II].
An 18-year old man presented with increasing pigmentation in multiple nevi as well as so-called "eruptive nevi" over a 24 month period. The first excisional biopsy was from a plantar nevus which showed melanocytic hyperplasia. Following excisions of both hyperpigmented and newly appearing nevi showed only increased pigmentation. Two years after the first examination Addison-like hyperpigmentation was noticed in typical locations. Both Addison disease and Hashimoto thyroiditis were then confirmed by elevated serum levels of ACTH, TSH, and decreased levels of cortisol and L-thyroxine. This confirmed the diagnosis of Schmidt syndrome as part of polyglandular autoimmune disease (PGAS) type II. After another 18 months of treatment with hormone substitution of L-thyroxine and hydrocortisone, the hyperpigmentation resolved and the hyperpigmented nevi decreased in number.
Sünkel
S
S
Ludwig-Maximilians-Universität München Klinikum Innenstadt, Medizinische Klinik, Endokrinologische Ambulanz.
Wichmann-Hesse
A
A
Gärtner
R
R
Hesse
G
G
D000224:Addison Disease / Q000150:complications; D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000627:therapeutic use; D001706:Biopsy; D004359:Drug Therapy, Combination; D005438:Fludrocortisone / Q000008:administration & dosage / Q000627:therapeutic use; D005500:Follow-Up Studies; D006801:Humans; D006854:Hydrocortisone / Q000008:administration & dosage / Q000627:therapeutic use; D017495:Hyperpigmentation / Q000188:drug therapy / Q000473:pathology; D008297:Male; D009508:Nevus, Pigmented / Q000150:complications / Q000473:pathology; D016884:Polyendocrinopathies, Autoimmune; D012867:Skin / Q000473:pathology; D013967:Thyroiditis, Autoimmune / Q000150:complications; D013974:Thyroxine / Q000008:administration & dosage / Q000627:therapeutic use; D013997:Time Factors
D000893:Anti-Inflammatory Agents; D013974:Thyroxine; D005438:Fludrocortisone; D006854:Hydrocortisone